Immune Thrombocytopenic Purpura Clinical Presentation

  • Author: Craig M Kessler, MD; Chief Editor: Emmanuel C Besa, MD   more...
 
Updated: Jan 3, 2012
 

History

The medical history in a patient with a clinical suspicion of immune thrombocytopenic purpura (ITP) should focus on (1) factors that suggest another disease for which thrombocytopenia is a complication[13] and (2) signs and symptoms that differentiate mild, moderate, and severe bleeding tendencies.

  • Other systemic illnesses
    • In adults, thrombocytopenic purpura may be a manifestation of systemic lupus erythematosus[14] or acute or chronic leukemia.
    • Thrombocytopenic purpura may be a manifestation of a myelodysplastic syndrome, particularly in patients older than 60 years.
    • In young children, immune thrombocytopenic purpura (ITP) may manifest as a primary immune deficiency syndrome.
  • Postviral illness
    • In children, most cases of immune thrombocytopenic purpura (ITP) are acute, and onset seems to occur within a few weeks of recovery from a viral illness. The severity of symptoms of the viral illness is not correlated with the degree of thrombocytopenia.
    • Thrombocytopenia is a recognized complication after infection with Epstein-Barr virus, varicella virus, cytomegalovirus, rubella virus, or hepatitis virus (A, B, or C), although the most typical association is a vaguely defined, viral, upper respiratory infection, or gastroenteritis.
    • Transient thrombocytopenia has been reported to be associated with recent immunization with attenuated live-virus vaccines.[15, 16]
  • Human immunodeficiency virus (HIV) infection
    • Thrombocytopenia may occur during the acute retroviral syndrome coincident with fever, rash, and sore throat.
    • Thrombocytopenia may be a manifestation of acquired immunodeficiency virus syndrome (AIDS), occurring late in the course of HIV infection.
    • Thrombocytopenia not uncommonly marks the onset of symptomatic HIV infection, particularly in people who abuse drugs.
  • Drug-induced thrombocytopenia
    • Regard any medication taken by a person who develops thrombocytopenia as a potential causative agent. A history of all prescription and over-the-counter medications is required to exclude drug-related thrombocytopenia.[17]
    • More than 1444 currently approved drugs are listed in the US Food and Drug Administration's Adverse Event Reporting System (AERS) database, all of which have been suspected of causing clinical episodes of thrombocytopenia. However, only 573 of these agents have a statistically significant reporting association with thrombocytopenia and of these, perhaps only two dozen satisfy clinical and laboratory criteria for evidence of causality for drug-induced thrombocytopenia.
    • A medication suspected of causing thrombocytopenia should exhibit a strict temporal relationship with the development of the low platelet count; the platelet number should recover when the offending medication is discontinued; the likelihood of drug-induced thrombocytopenia should be greater than any other plausible cause; and ideally, in vitro evidence of drug-dependent antibody formation should exist. Reese et al have published a useful online database of the drugs most likely to cause thrombocytopenia.[18]
    • Persons who have been sensitized (by previous exposure) to quinidine or quinine may develop immune-mediated drug purpura within hours to days of subsequent exposure. To exclude drug purpura in a person previously treated with quinidine or quinine, the history must include questions about possible exposure to over-the-counter medications, tonic water in cocktails, or bitter lemon beverages.
    • Investigate the records of patients who have been hospitalized and who develop acute thrombocytopenias for all of their medications that are listed and not listed in nursing charts. For example, people who are at risk for heparin-induced thrombocytopenia because of current or recent treatment with heparin may be receiving the heparin with the routine flushing of intravenous (IV) catheters, and this exposure may not be listed on the nursing medication sheet. Many catheters are also heparin impregnated, and unless checked, they can be a hidden cause of heparin-induced thrombocytopenia.
    • Other drugs associated with drug purpura include antibiotics (eg, cephalosporins, rifampicin), gold salts, analgesics, neuroleptics, diuretics, antihypertensives, eptifibatide (Integrilin), and abciximab (ReoPro), which is a Fab fragment of the chimeric human-murine monoclonal antibody 7E3 directed against the platelet GPIIb/IIIa receptor.
    • Acute and chronic alcohol consumption may also be associated with thrombocytopenia. In persons with chronic liver disease, hypersplenism with secondary thrombocytopenia is not uncommon.
  • Bleeding tendency
    • Determine the extent and duration of the bleeding tendency to estimate the severity of the illness and the potential risk for a serious hemorrhage. Previous surgical history can often provide a useful clue regarding the acuteness of thrombocytopenia.
    • Query patients to elicit signs or symptoms of intracranial bleeding, such as headache, blurred vision, somnolence, or loss of consciousness.
    • Patients should report any recent accidental head trauma.
    • Record any bleeding, including petechiae, ecchymoses, epistaxis, menorrhagia, melena, or hematuria. Determine if bruising or bleeding is a recurrent problem.
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Physical

Similar to the medical history, focus the physical examination on (1) findings that suggest another disease for which thrombocytopenia is a complication and (2) physical signs that suggest serious internal bleeding.

  • General health
    • Immune thrombocytopenic purpura (ITP) is a primary illness occurring in an otherwise healthy person.
    • Signs of chronic disease, infection, wasting, or poor nutrition indicate that the patient has another illness.
  • Vital signs: Hypertension and bradycardia may be signs of increased intracranial pressure and evidence of an undiagnosed intracranial hemorrhage.
  • Skin and mucous membranes
    • An initial impression of the severity of immune thrombocytopenic purpura (ITP) is formed by examining the skin and mucous membranes.
    • Widespread petechiae and ecchymoses, oozing from a venipuncture site, gingival bleeding, and hemorrhagic bullae indicate that the patient is at risk for a serious bleeding complication. If the patient's blood pressure was taken recently, petechiae may be observed under and distal to the area where the cuff was placed and inflated. Suction-type electrocardiograph (ECG) leads may similarly induce petechiae.
    • Mild thrombocytopenia and a relatively low risk for a serious bleeding complication may manifest as petechiae over the ankles in patients who are ambulatory or on the back in patients who are bedridden.
  • Cardiovascular system: Distant low-amplitude heart sounds accompanied by jugular venous distention may be evidence of hemopericardium.
  • Abdomen
    • In children with acute immune thrombocytopenic purpura (ITP), the presence of a readily palpable spleen is not typical.
    • In an adult, hepatosplenomegaly is also atypical for immune thrombocytopenic purpura (ITP) and may indicate chronic liver and other diseases. In fact, splenomegaly excludes the diagnosis of immune thrombocytopenic purpura (ITP).
  • Nervous system
    • Any asymmetrical finding of recent onset can indicate an intracranial hemorrhage.
    • Pupils should be equal in size and have intact extraocular muscles and symmetrical eye movements.
    • Balance and gait should be intact.
    • Funduscopic examination reveals whether the margins of the optic disc are blurred. Examine the patient for the presence of retinal hemorrhages and other evidence of increased intracranial pressure.
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Causes

In children, most cases of immune thrombocytopenic purpura (ITP) are acute, manifesting a few weeks after a viral illness. In adults, most cases of immune thrombocytopenic purpura (ITP) are chronic, manifesting with an insidious onset, typically in middle-aged women. These clinical presentations suggest that the triggering events may be different. However, in both children and adults, the cause of thrombocytopenia (destruction of [antibody-coated] immunoglobulin-coated platelets by mononuclear macrophages) appears to be similar.

  • Autoantibody stimulation
    • In persons with chronic immune thrombocytopenic purpura (ITP), membrane GPs on the surface of platelets become immunogenic, stimulating the production of platelet autoantibodies.
    • In persons with acute immune thrombocytopenic purpura (ITP), the stimulus for autoantibody production is also unknown. Platelet membrane cryptantigens may become exposed by the stress of infection, or pseudoantigens may be formed by the passive adsorption of pathogens on platelet surfaces.
  • Autoantibody specificity
    • In persons with chronic immune thrombocytopenic purpura (ITP), approximately 75% of autoantibodies are directed against platelet GPIIb/IIIa or GPIb/IX GP complexes.
    • Presumably, the remaining 25% are directed against other membrane epitopes, including GPV, GPIa/IIa, or GPIV.
  • Role of the spleen[5]
    • The spleen is the site of autoantibody production (white pulp).
    • It is also the site of phagocytosis of autoantibody-coated platelets (red pulp).
    • The slow passage of platelets through splenic sinusoids with a high local concentration of antibodies and Fc-gamma receptors on splenic macrophages lend to the uniqueness of the spleen as a site of platelet destruction.
    • Low-affinity macrophage receptors, Fc gamma RIIA, and Fc gamma RIIIA bind immune-complexed IgG and are the key mediators of platelet clearance.
  • Platelet destruction
    • The mononuclear macrophage system of the spleen is responsible for removing platelets in immune thrombocytopenic purpura (ITP), because splenectomy results in prompt restoration of normal platelet counts in most patients with immune thrombocytopenic purpura (ITP).
    • Platelets are sequestered and destroyed by mononuclear macrophages, which are neither reticular nor endothelial in origin. Therefore, the former designation of reticuloendothelial system is considered imprecise.
    • Immune destruction of immunoglobulin-coated platelets is mediated by macrophage IgG Fc (Fc gamma RI, Fc gamma RII, and Fc gamma RIII) and complement receptors (CR1, CR3).
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Contributor Information and Disclosures
Author

Craig M Kessler, MD  Professor, Department of Medicine and Pathology, Division of Hematology/Oncology, Georgetown University School of Medicine; Director, Clinical Coagulation Laboratory, Lombardi Comprehensive Cancer Center, Georgetown University Hospital

Disclosure: NovoNordisk Honoraria Consulting; NovoNordisk Grant/research funds Other; Baxter-Immuno Honoraria Consulting

Coauthor(s)

S Gerald Sandler, MD, FACP, FCAP  Professor of Medicine and Pathology, Director, Transfusion Medicine, Department of Laboratory Medicine, Georgetown University Hospital

S Gerald Sandler, MD, FACP, FCAP is a member of the following medical societies: American Association of Blood Banks, College of American Pathologists, and International Society of Blood Transfusions

Disclosure: Nothing to disclose.

Rumina Bhanji, MD  Staff Physician, Departments of Pathology and Laboratory Medicine, Georgetown University Hospital

Disclosure: Nothing to disclose.

Specialty Editor Board

Michael Paul Kosty, MD  Associate Director, Associate Professor, Department of Internal Medicine, Divisions of Supportive Care Services and Hematology and Oncology, Ida M and Cecil H Green Cancer Center, Scripps Clinic

Michael Paul Kosty, MD is a member of the following medical societies: American College of Physicians, American Society of Hematology, and Phi Beta Kappa

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Ronald A Sacher, MB, BCh, MD, FRCPC  Professor, Internal Medicine and Pathology, Director, Hoxworth Blood Center, University of Cincinnati Academic Health Center

Ronald A Sacher, MB, BCh, MD, FRCPC is a member of the following medical societies: American Association for the Advancement of Science, American Association of Blood Banks, American Clinical and Climatological Association, American Society for Clinical Pathology, American Society of Hematology, College of American Pathologists, International Society of Blood Transfusion, International Society on Thrombosis and Haemostasis, and Royal College of Physicians and Surgeons of Canada

Disclosure: Glaxo Smith Kline Honoraria Speaking and teaching; Talecris Honoraria Board membership

Rajalaxmi McKenna, MD, FACP  Southwest Medical Consultants, SC, Department of Medicine, Good Samaritan Hospital, Advocate Health Systems

Rajalaxmi McKenna, MD, FACP is a member of the following medical societies: American Society of Clinical Oncology, American Society of Hematology, and International Society on Thrombosis and Haemostasis

Disclosure: Nothing to disclose.

Chief Editor

Emmanuel C Besa, MD  Professor, Department of Medicine, Division of Hematologic Malignancies, Kimmel Cancer Center, Jefferson Medical College of Thomas Jefferson University

Emmanuel C Besa, MD is a member of the following medical societies: American Association for Cancer Education, American College of Clinical Pharmacology, American Federation for Medical Research, American Society of Clinical Oncology, American Society of Hematology, and New York Academy of Sciences

Disclosure: Nothing to disclose.

Additional Contributors

The authors and editors of eMedicine gratefully acknowledge the contributions of previous author S Gerald Sandler, MD, FACP, FCAP, to the development and writing of this article.

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Peripheral blood smear from a patient with immune thrombocytopenic purpura (ITP) illustrates a decreased number of platelets, a normal-appearing neutrophil, and erythrocytes. ITP is diagnosed by excluding other diseases; therefore, the absence of other findings from the peripheral smear is at least as important as the findings are observed. This smear demonstrates the absence of immature leukocytes (as in leukemia) and fragmented erythrocytes (as in thrombotic thrombocytopenic purpura) and no clumps of platelets (as in pseudothrombocytopenia).
 
 
 
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